BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon malignant neoplasm of the upper nasal cavity. Therapeutic management approaches for this neoplasm lack uniformity and there is no universally accepted staging system. METHODS: A retrospective review of 27 patients with histologically confirmed ENB managed at The Johns Hopkins Hospital. RESULTS: Eighty-five percent of patients had surgical resection as part of their disease management. Complete surgical resection was achieved in 62% of patients who had a craniofacial resection. Eighty percent of patients with negative surgical margins remain with no evidence of disease, with a median follow-up of 5.6 years. Adjuvant radiation therapy was beneficial to 62% of patients with positive surgical margins. Clinical responses were observed with cisplatin- and etoposide-containing chemotherapy regimens in patients with advanced disease. A revised staging system based on our experience is proposed. CONCLUSIONS: ENB is best managed by craniofacial resection with complete tumor resection. Adjuvant radiation therapy is warranted in patients that remain with positive histologic margins of resection. Chemotherapy with cisplatin- and etoposide-containing regimens may be useful for palliation of advanced disease.
BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon malignant neoplasm of the upper nasal cavity. Therapeutic management approaches for this neoplasm lack uniformity and there is no universally accepted staging system. METHODS: A retrospective review of 27 patients with histologically confirmed ENB managed at The Johns Hopkins Hospital. RESULTS: Eighty-five percent of patients had surgical resection as part of their disease management. Complete surgical resection was achieved in 62% of patients who had a craniofacial resection. Eighty percent of patients with negative surgical margins remain with no evidence of disease, with a median follow-up of 5.6 years. Adjuvant radiation therapy was beneficial to 62% of patients with positive surgical margins. Clinical responses were observed with cisplatin- and etoposide-containing chemotherapy regimens in patients with advanced disease. A revised staging system based on our experience is proposed. CONCLUSIONS: ENB is best managed by craniofacial resection with complete tumor resection. Adjuvant radiation therapy is warranted in patients that remain with positive histologic margins of resection. Chemotherapy with cisplatin- and etoposide-containing regimens may be useful for palliation of advanced disease.
Authors: Marc W Herr; Stacey T Gray; Audrey B Erman; William T Curry; Daniel G Deschler; Derrick T Lin Journal: J Neurol Surg B Skull Base Date: 2013-03-13
Authors: Paolo G Castelnuovo; Giovanni Delù; Federica Sberze; Andrea Pistochini; Cristhian Cambria; Paolo Battaglia; Maurizio Bignami Journal: Skull Base Date: 2006-02
Authors: Rohan R Joshi; Qasim Husain; Benjamin R Roman; Jennifer Cracchiolo; Yao Yu; Jillian Tsai; Julie Kang; Sean McBride; Nancy Y Lee; Luc Morris; Ian Ganly; Viviane Tabar; Marc A Cohen Journal: J Surg Oncol Date: 2018-11-22 Impact factor: 3.454